Table 1.
Selected patient, medical, and laboratory testing characteristics (n = 325).
| Variable | n | Value* |
|---|---|---|
| Medical history (n = 56 variables, selected variables shown) | ||
| Age at enrolment (years) | 325 | 13.6 (11.5, 16.0) |
| Male gender | 325 | 193 (59%) |
| Ethnicity | 325 | |
| White | 270 (84%) | |
| Black | 32 (10%) | |
| Asian | 7 (2%) | |
| Other | 16 (5%) | |
| Cardiac anatomy | 325 | |
| Tricuspid atresia | 80 (25%) | |
| Hypoplastic left heart syndrome | 58 (18%) | |
| Double-inlet left ventricle | 53 (16%) | |
| Heterotaxia | 22 (7%) | |
| Mitral atresia | 18 (5%) | |
| Unbalanced atrioventricular septal defect | 12 (4%) | |
| Other single ventricle | 76 (23%) | |
| Age at Fontan procedure (years) | 325 | 2.9 (2.2, 4.4) |
| Fontan connection type | 325 | |
| Intracardiac lateral tunnel | 196 (60%) | |
| Atriopulmonary connection | 70 (22%) | |
| Extracardiac conduit | 48 (15%) | |
| Extracardiac lateral tunnel | 1 (<%) | |
| Other | 10 (3%) | |
| Years since Fontan procedure | 325 | 10.5 (8.3, 12.4) |
| Post-Fontan morbidities | 325 | |
| Arrhythmia | 73 (23%) | |
| Ventricular dysfunction | 43 (13%) | |
| Thrombosis | 21 (7%) | |
| Protein-losing enteropathy | 12 (4%) | |
| Stroke | 9 (3%) | |
| Current cardiac medication use | 325 | 189 (58%) |
| Number of current cardiac medications | 189 | 2 (1, 3) |
| Laboratory testing | ||
| Brain natriuretic peptide (pg/ml) | 309 | 13 (7, 29) |
| Echocardiography (n = 16 variables, selected variables shown) | ||
| Ejection fraction (%) | 245 | 58±11 |
| Ejection fraction z-score | 245 | 20.9±2.1 |
| Ventricular end-diastolic volume (ml) | 245 | 94±46 |
| Ventricular end-diastolic volume z-score | 245 | 20.7±1.9 |
| Ventricular mass (g) | 240 | 110±52 |
| Ventricular mass z-score | 240 | 1.0±2.4 |
| Ventricular mass/volume ratio (g/ml) | 240 | 1.2±0.4 |
| Ventricular mass/volume ratio z-score | 240 | 2.8±3.3 |
| Cardiopulmonary exercise testing (n = 6 variables) | ||
| Chronotropic index | 289 | 0.6±0.2 |
| Resting systemic oxygen saturation (%) | 288 | 94±4 |
| Percentage predicted peak oxygen consumption | 287 | 63±15 |
| Percentage predicted maximum work rate | 288 | 62±17 |
| Percentage predicted oxygen consumption at anaerobic threshold | 245 | 76±22 |
| Percentage predicted maximum oxygen pulse | 287 | 87±22 |
| Magnetic resonance imaging (n = 7 variables) | ||
| Total stroke volume (ml) | 109 | 72±23 |
| Total ejection fraction (%) | 109 | 56±10 |
| Total cardiac output (l/minute) | 107 | 5.5±1.8 |
| Total indexed end-systolic volume (ml/m2) | 109 | 36±14 |
| Total indexed end-diastolic volume (ml/m2) | 109 | 81±21 |
| Total indexed ventricular mass (g/m2) | 109 | 70±20 |
| Total mass/end-diastolic volume ratio (g/ml) | 109 | 0.9±0.3 |
*
Values represent frequency (%), median (25th, 75th percentiles), or mean (±standard deviation)